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Risk factors for right ventricular dysfunction in patients with lymphangioleiomyomatosis

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Abstract

Lymphangioleiomyomatosis (LAM) is a rare disease characterized by diffuse cystic lesions of the lung. The present study was designed to evaluate the right ventricular (RV) function in LAM patients via single-beat real-time three-dimensional echocardiography (RT-3DE) and to investigate the factors affecting RV function in LAM patients. According to tricuspid regurgitation velocity (TRV), forty-five female LAM patients [(44.07 ± 10.22) years old] were divided into TRV ≤ 2.8 m/s group (n = 29) and TRV > 2.8 m/s group (n = 16). Relative echocardiography parameters were assessed by conventional transthoracic echocardiography, Doppler tissue imaging (DTI) and RT-3DE, respectively. Pulmonary function tests and the six-minute walk tests (SMWT) were also performed for LAM patients. We found that most of RV functional parameters in LAM patients were worse than that in control patients, although left ventricular dysfunction was not significantly observed. Correlation analysis showed that 3D echocardiographic RV ejection fraction (RVEF) was negatively correlated with pulmonary vascular resistance (PVR), TRV, and the decrease of oxygen saturation (SpO2) post SMWT, and positively correlated with Forced expiratory volume in the first second/forced vital capacity, carbon monoxide diffusion predicted value, SMWT distance, and resting SpO2 in LAM patients. Multivariate stepwise linear regression analysis showed that PVR and SpO2 before SMWT were independent influence factors of RVEF in LAM patients. In this study, we found that RV dysfunction was presented in LAM patients, although left ventricular dysfunction was not significantly obvious. The main influence factors of RVEF were PVR and hypoxia. RT-3DE is a low-cost and noninvasive way to evaluate RV function in LAM patients.

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The datasets supporting the results of this article are included within the article.

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TomTec Right Ventricular Analysis.

References

  1. McCormack FX, Gupta N, Finlay GR et al (2016) Official American Thoracic Society/Japanese Respiratory Society Clinical Practice Guidelines: lymphangioleiomyomatosis diagnosis and management. Am J Respir Crit Care Med 194(6):748–761. https://doi.org/10.1164/rccm.201607-1384ST

    Article  PubMed  PubMed Central  Google Scholar 

  2. Gupta N, Lee HS, Ryu JH, Taveira-DaSilva AM, Beck GJ, Lee JC, McCarthy K, Finlay GA, Brown KK, Ruoss SJ, Avila NA, Moss J, McCormack FX (2019) The NHLBI LAM Registry: prognostic physiologic and radiologic biomarkers emerge from a 15-year prospective longitudinal analysis. Chest 155(2):288–296. https://doi.org/10.1016/j.chest.2018.06.016

    Article  PubMed  Google Scholar 

  3. Harari S, Torre O, Cassandro R, Moss J (2015) The changing face of a rare disease: lymphangioleiomyomatosis. Eur Respir J 46(5):1471–1485. https://doi.org/10.1183/13993003.00412-2015

    Article  CAS  PubMed  Google Scholar 

  4. Zhang L, Wang MJ, Wang W, Zhao JY, Wu JL, Liu YP, Zhu H, Qu JM, Zhou M (2019) Identification of driver genes and somatic mutations in cell-free DNA of patients with pulmonary lymphangioleiomyomatosis. Int J Cancer. https://doi.org/10.1002/ijc.32511

    Article  PubMed  PubMed Central  Google Scholar 

  5. Johnson SR, Taveira-DaSilva AM, Moss J (2016) Lymphangioleiomyomatosis. Clin Chest Med 37(3):389–403. https://doi.org/10.1016/j.ccm.2016.04.002

    Article  PubMed  Google Scholar 

  6. Amsallem M, Mercier O, Kobayashi Y, Moneghetti K, Haddad F (2018) Forgotten no more: a focused update on the right ventricle in cardiovascular disease. JACC Heart Fail 6(11):891–903. https://doi.org/10.1016/j.jchf.2018.05.022

    Article  PubMed  Google Scholar 

  7. Rivera-Lebron BN, Forfia PR, Kreider M, Lee JC, Holmes JH, Kawut SM (2013) Echocardiographic and hemodynamic predictors of mortality in idiopathic pulmonary fibrosis. Chest 144(2):564–570. https://doi.org/10.1378/chest.12-2298

    Article  PubMed  PubMed Central  Google Scholar 

  8. Zangiabadi A, De Pasquale CG, Sajkov D (2014) Pulmonary hypertension and right heart dysfunction in chronic lung disease. BioMed Res Int 2014:739674. https://doi.org/10.1155/2014/739674

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  9. Freixa X, Portillo K, Pare C, Garcia-Aymerich J, Gomez FP, Benet M, Roca J, Farrero E, Ferrer J, Fernandez-Palomeque C, Anto JM, Barbera JA (2013) Echocardiographic abnormalities in patients with COPD at their first hospital admission. Eur Respir J 41(4):784–791. https://doi.org/10.1183/09031936.00222511

    Article  PubMed  Google Scholar 

  10. Hilde JM, Skjorten I, Grotta OJ, Hansteen V, Melsom MN, Hisdal J, Humerfelt S, Steine K (2013) Right ventricular dysfunction and remodeling in chronic obstructive pulmonary disease without pulmonary hypertension. J Am Coll Cardiol 62(12):1103–1111. https://doi.org/10.1016/j.jacc.2013.04.091

    Article  PubMed  Google Scholar 

  11. D'Andrea A, Stanziola A, Di Palma E, Martino M, D'Alto M, Dellegrottaglie S, Cocchia R, Riegler L, Betancourt Cordido MV, Lanza M, Maglione M, Diana V, Calabro R, Russo MG, Vannan M, Bossone E (2016) Right ventricular structure and function in idiopathic pulmonary fibrosis with or without pulmonary hypertension. Echocardiography (Mount Kisco, NY) 33(1):57–65. https://doi.org/10.1111/echo.12992

    Article  Google Scholar 

  12. Park JB, Lee SP, Lee JH, Yoon YE, Park EA, Kim HK, Lee W, Kim YJ, Cho GY, Sohn DW (2016) Quantification of right ventricular volume and function using single-beat three-dimensional echocardiography: a validation study with cardiac magnetic resonance. J Am Soc Echocardiogr 29(5):392–401. https://doi.org/10.1016/j.echo.2016.01.010

    Article  PubMed  Google Scholar 

  13. Knight DS, Grasso AE, Quail MA, Muthurangu V, Taylor AM, Toumpanakis C, Caplin ME, Coghlan JG, Davar J (2015) Accuracy and reproducibility of right ventricular quantification in patients with pressure and volume overload using single-beat three-dimensional echocardiography. J Am Soc Echocardiogr 28(3):363–374. https://doi.org/10.1016/j.echo.2014.10.012

    Article  PubMed  PubMed Central  Google Scholar 

  14. Johnson SR, Cordier JF, Lazor R, Cottin V, Costabel U, Harari S, Reynaud-Gaubert M, Boehler A, Brauner M, Popper H, Bonetti F, Kingswood C, Review Panel of the ERSLAMTF (2010) European Respiratory Society guidelines for the diagnosis and management of lymphangioleiomyomatosis. Eur Respir J 35(1):14–26. https://doi.org/10.1183/09031936.00076209

    Article  CAS  PubMed  Google Scholar 

  15. Lang RM, Badano LP, Mor-Avi V, Afilalo J, Armstrong A, Ernande L, Flachskampf FA, Foster E, Goldstein SA, Kuznetsova T, Lancellotti P, Muraru D, Picard MH, Rietzschel ER, Rudski L, Spencer KT, Tsang W, Voigt JU (2015) Recommendations for cardiac chamber quantification by echocardiography in adults: an update from the American Society of Echocardiography and the European Association of Cardiovascular Imaging. J Am Soc Echocardiogr 28(1):1–39. https://doi.org/10.1016/j.echo.2014.10.003

    Article  PubMed  Google Scholar 

  16. Torbicki A, Peacock A, Vonk Noordegraaf A, Ghofrani A, Hansmann G, Simonneau G, Lang I, Vachiery J-L, Pierard LA, Matucci M, Hoeper M, Beghetti M, Zompatori M, Gomez Sanchez MA, Lancellotti P, Trindade PT, Gibbs S, McDonagh T, Klepetko W, Humbert M, Galiè N, Group ESD (2015) 2015 ESC/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension: the Joint Task Force for the Diagnosis and Treatment of Pulmonary Hypertension of the European Society of Cardiology (ESC) and the European Respiratory Society (ERS): Endorsed by: Association for European Paediatric and Congenital Cardiology (AEPC), International Society for Heart and Lung Transplantation (ISHLT). Eur Heart J 37(1):67–119. https://doi.org/10.1093/eurheartj/ehv317

    Article  PubMed  Google Scholar 

  17. Abbas AE, Fortuin FD, Schiller NB, Appleton CP, Moreno CA, Lester SJ (2003) A simple method for noninvasive estimation of pulmonary vascular resistance. J Am Coll Cardiol 41(6):1021–1027

    Article  Google Scholar 

  18. Sonaglioni A, Baravelli M, Cassandro R, Torre O, Elia D, Anza C, Harari S (2018) Hemodynamic mechanisms of exercise-induced pulmonary hypertension in patients with lymphangioleiomyomatosis: the role of exercise stress echocardiography. J Am Soc Echocardiogr 31(8):888–901. https://doi.org/10.1016/j.echo.2018.02.004

    Article  PubMed  Google Scholar 

  19. Freitas CSG, Baldi BG, Jardim C, Araujo MS, Sobral JB, Heiden GI, Kairalla RA, Souza R, Carvalho CRR (2017) Pulmonary hypertension in lymphangioleiomyomatosis: prevalence, severity and the role of carbon monoxide diffusion capacity as a screening method. Orphanet J Rare Dis 12(1):74. https://doi.org/10.1186/s13023-017-0626-0

    Article  PubMed  PubMed Central  Google Scholar 

  20. Cottin V, Harari S, Humbert M, Mal H, Dorfmuller P, Jais X, Reynaud-Gaubert M, Prevot G, Lazor R, Taille C, Lacronique J, Zeghmar S, Simonneau G, Cordier JF (2012) Pulmonary hypertension in lymphangioleiomyomatosis: characteristics in 20 patients. Eur Respir J 40(3):630–640. https://doi.org/10.1183/09031936.00093111

    Article  PubMed  Google Scholar 

  21. Taveira-DaSilva AM, Hathaway OM, Sachdev V, Shizukuda Y, Birdsall CW, Moss J (2007) Pulmonary artery pressure in lymphangioleiomyomatosis: an echocardiographic study. Chest 132(5):1573–1578. https://doi.org/10.1378/chest.07-1205

    Article  PubMed  PubMed Central  Google Scholar 

  22. Tannus-Silva DG, Rabahi MF (2017) State of the art review of the right ventricle in COPD patients: it is time to look closer. Lung 195(1):9–17. https://doi.org/10.1007/s00408-016-9961-5

    Article  PubMed  Google Scholar 

  23. Rowan SC, Keane MP, Gaine S, McLoughlin P (2016) Hypoxic pulmonary hypertension in chronic lung diseases: novel vasoconstrictor pathways. Lancet Respir Med 4(3):225–236. https://doi.org/10.1016/s2213-2600(15)00517-2

    Article  PubMed  Google Scholar 

  24. Dunham-Snary KJ, Wu D, Sykes EA, Thakrar A, Parlow LRG, Mewburn JD, Parlow JL, Archer SL (2017) Hypoxic pulmonary vasoconstriction: from molecular mechanisms to medicine. Chest 151(1):181–192. https://doi.org/10.1016/j.chest.2016.09.001

    Article  PubMed  Google Scholar 

  25. Kovacs A, Lakatos B, Tokodi M, Merkely B (2019) Right ventricular mechanical pattern in health and disease: beyond longitudinal shortening. Heart Fail Rev. https://doi.org/10.1007/s10741-019-09778-1

    Article  PubMed  PubMed Central  Google Scholar 

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Funding

This work was funded by Shanghai Three-year Plan of Action for Further Accelerating the Development of Traditional Chinese Medicine (2018-2020) [NO. ZY (2018-2020)-FWTX-1003], Shanghai Comprehensive Hospital Integrated Chinese and Western Medicine Project (ZHYY-ZXYJHZX-1-06) and Shanghai Municipal Education Commission – Gaofeng Clinical Medicine Grant Support (20161402).

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Contributions

YF, JQ and MZ conceived and designed the study. WH, WY, JG, JW, WW, YL and HZ collected the data and performed analysis. WH and WY drafted manuscript. All authors critically reviewed data analysis and the manuscript. All authors read and approved the final manuscript.

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Correspondence to Jieming Qu.

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The authors declare that they have no conflict of interest.

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All procedures performed in studies involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards. The study was approved by the Ethics Committee of Ruijin Hospital Affiliated with Shanghai Jiaotong University School of Medicine.

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Hua, W., Yang, W., Gu, J. et al. Risk factors for right ventricular dysfunction in patients with lymphangioleiomyomatosis. Int J Cardiovasc Imaging 37, 439–448 (2021). https://doi.org/10.1007/s10554-020-02009-x

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